Abstract
Hemostasis is a complex and tightly regulated process whereby the body attempts to maintain a homeostatic balance to permit normal blood flow, without bleeding or thrombosis. When this balance is disrupted, due to trauma or underlying congenital bleeding or thrombotic disorders, clinical intervention may be required. To assist clinicians in diagnosing and managing affected patients, hemostasis laboratories offer an arsenal of tests, both routine (screening) and more specialized (diagnostic). In general, screening assays are used to screen for hemostasis-related disease or to monitor or measure the effect of anticoagulant therapy, which may be applied to treat patients with recent thrombosis or at risk of thrombosis. Diagnostic assays are used to diagnose or exclude specific hemostasis-related diseases, and in some cases, to monitor or measure the effect of anticoagulant therapy, or alternatively procoagulant therapy that may be applied to those at risk of bleeding. This chapter provides an overview of hemostasis and thrombosis with respect to laboratory tests that may be applied to affected patients.
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References
Lippi G, Favaloro EJ, Franchini M, Guidi GC (2009) Milestones and perspectives in coagulation and hemostasis. Semin Thromb Hemost 35:9–22
Lippi G, Franchini M, Favaloro EJ (2016) Diagnostics of inherited bleeding disorders of secondary haemostasis: an easy guide for routine clinical laboratories. Semin Thromb Hemost 42:471–477
Lippi G, Pasalic L, Favaloro EJ (2015) Detection of mild inherited disorders of blood coagulation: current options and personal recommendations. Expert Rev Hematol 8:527–542
Bonar RA, Favaloro EJ (2010) Quality in coagulation and haemostasis testing. Biochem Med 20:184–199
Sadler JE, Budde U, Eikenboom JC, et al.; Working party on von Willebrand Disease Classification (2006) Update on the pathophysiology and classification of von Willebrand Disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 4, 2103–2114
Blanchette VS, Srivastava A (2015) Definitions in hemophilia: resolved and unresolved issues. Semin Thromb Hemost 41:819–825
Favaloro EJ, Pasalic L, Curnow J (2016) Laboratory tests used to help diagnose von Willebrand disease: an update. Pathology 48:303–318
Favaloro EJ, Bodó I, Israels SJ, Brown SA (2014) von Willebrand disease and platelet disorders. Haemophilia 20(Suppl 4):59–64
Coppola A, Tufano A, Cerbone AM, Di Minno G (2009) Inherited thrombophilia: implications for prevention and treatment of venous thromboembolism. Semin Thromb Hemost 35:683–694
Favaloro EJ (2015) Anticoagulant therapy: present and future. Semin Thromb Hemost 41:109–112
Favaloro EJ, Lippi G (2015) Laboratory testing in the era of direct or non-vitamin k antagonist oral anticoagulants: a practical guide to measuring their activity and avoiding diagnostic errors. Semin Thromb Hemost 41:208–227
Favaloro EJ, Lippi G (2017) Interference of direct oral anticoagulants (DOACs) in hemostasis assays: high potential for diagnostic false positives and false negatives. Blood Transfus 7:1–4
Favaloro EJ, Lippi G, Adcock DM (2008) Preanalytical and postanalytical variables: the leading causes of diagnostic error in haemostasis. Semin Thromb Hemost 34:612–634
Adcock DM, Favaloro EJ, Lippi G (2016) Critical pre-examination variables in the hemostasis laboratory and their quality indicators. Critical pre-examination variables in the hemostasis laboratory and their quality indicators. Clin Biochem 49:1315–1320
Favaloro EJ, Lippi G (2010) Laboratory reporting of hemostasis assays: the final post-analytical opportunity to reduce errors of clinical diagnosis in hemostasis? Clin Chem Lab Med 48:309–321
Lippi G, Franchini M, Guidi GC (2007) Diagnostic approach to inherited bleeding disorders. Clin Chem Lab Med 45:2–12
Yee A, Kretz CA (2014) Von Willebrand Factor: form for function. Semin Thromb Hemost 40:17–27
Kwaan H, Lisman T, Medcalf RL (2017) Fibrinolysis: biochemistry, clinical aspects, and therapeutic potential. Semin Thromb Hemost 43(2):113–114
Hoffman M (2003) A cell-based model of coagulation and the role of factor VIIa. Blood Rev 17:51–55
Quick AJ (1935) The prothrombin time in haemophilia and in obstructive jaundice. J Biol Chem 109:73–74
Favaloro EJ, Adcock DM (2008) Standardisation of the INR: how good is your laboratory’s INR and can it be improved. Semin Thromb Hemost 34:593–603
Bonar R, Mohammed S, Favaloro EJ (2015) International normalized ratio monitoring of vitamin K antagonist therapy: comparative performance of point-of-care and laboratory-derived testing. Semin Thromb Hemost 41:279–286
Kitchen DP, Jennings I, Kitchen S, Woods TA, Walker ID (2015) Bridging the gap between point-of-care testing and laboratory testing in hemostasis. Semin Thromb Hemost 41:272–278
Bonar R, Favaloro EJ (2017) Explaining and reducing the variation in inter-laboratory reported values for International Normalised Ratio. Thromb Res 150:22–29
Lippi G, Favaloro EJ (2008) Activated partial thromboplastin time: new tricks for an old dogma. Semin Thromb Hemost 34:604–611
Pengo V, Tripodi A, Reber G et al (2009) Update of the guidelines for lupus anticoagulant detection. J Thromb Haemost 7:1737–1740
Jennings I, Kitchen S, Kitchen DP, Woods TAL, Walker ID (2011) ISTH/SSC lupus anticoagulant testing guidelines: how far have these been adopted by laboratories? J Thromb Haemost 9:2117–2179
Penmetsa GK, Rodgers GM, Smock KJ (2010) Avoiding errors in the laboratory evaluation of potent lupus anticoagulants. Am J Hematol 85:272–274
Undas A (2016) How to assess fibrinogen levels and fibrin clot properties in clinical practice? Semin Thromb Hemost 42:381–388
Muszbek L, Katona É (2016) Diagnosis and management of congenital and acquired FXIII deficiencies. Semin Thromb Hemost 42:429–439
Lippi G, Danese E, Favaloro EJ, Montagnana M, Franchini M (2015) Diagnostics in venous thromboembolism: from origin to future prospects. Semin Thromb Hemost 41:374–381
Favaloro EJ, Bonar R, Sioufi J, Wheeler M, Low J, Aboud M, Duncan E, Smith J, Exner T, Lloyd J, Marsden K (2005) Multi-laboratory testing of thrombophilia: current and past practice in Austrasia as assessed through the Royal College of Pathologist of Australasia quality Assurance Program for Hematology. Semin Thromb Hemost 31:49–58
Favaloro EJ, Wong RCW (2014) Antiphospholipid antibody testing for the antiphospholipid syndrome: a synopsis of challenges and recent guidelines. Pathology 46:481–495
Galli M, Luciani D, Bertolini G, Barbui T (2003) Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood 101:1827–1832
Moore GW (2014) Recent guidelines and recommendations for laboratory detection of lupus anticoagulants. Semin Thromb Hemost 40:163–171
Favaloro EJ, Bonar RA, Marsden K (2012) Internal quality control and external quality assurance in testing for antiphospholipid antibodies: part II-lupus anticoagulant. Semin Thromb Hemost 38:404–411
Bonar R, Favaloro E, Zebeljan D et al (2012) Evaluating laboratory approaches to the identification of lupus anticoagulants: a diagnostic challenge from the RCPA Haematology QAP. Pathology 44:240–247
Favaloro EJ, Lippi G, Koutts J (2011) Laboratory testing of anticoagulant: the present and future. Pathology 43:682–692
Lippi G, Favaloro EJ (2015) Recent guidelines and recommendations for laboratory assessment of the direct oral anticoagulants (DOACs): is there consensus? Clin Chem Lab Med 53:185–197
Cuker A (2012) Unfractionated heparin for the treatment of venous thromboembolism: best practices and areas of uncertainty. Semin Thromb Hemost 38:593–599
Warkentin TE (2015) Heparin-induced thrombocytopenia in critically ill patients. Semin Thromb Hemost 41:49–60
Cuker A (2014) Clinical and laboratory diagnosis of heparin-induced thrombocytopenia: an integrated approach. Semin Thromb Hemost 40:106–114
Bonar R, Favaloro EJ, Marsden K (2012) External quality assurance in heparin monitoring. Semin Thromb Hemost 38:632–639
Bonar R, Favaloro EJ, Mohammed S, Pasalic L, Sioufi J, Marsden K (2015) The effect of dabigatran on haemostasis tests: a comprehensive assessment using in-vitro and ex-vivo samples. Pathology 47:355–364
Bonar R, Favaloro EJ, Mohammed S, Ahuja M, Pasalic L, Sioufi J, Marsden K (2016) The effect of the direct factor Xa inhibitors apixaban and rivaroxaban on haemostasis tests: a comprehensive assessment using in vitro and ex vivo samples. Pathology 48:60–71
Favaloro EJ, Bonar RA, Meiring M, Duncan E, Mohammed S, Sioufi J, Marsden K (2014) Evaluating errors in the laboratory identification of von Willebrand disease in the real world. Thromb Res 134:393–403
Favaloro EJ, Bonar RA, Mohammed S, Arbelaez A, Niemann F, Freney R, Meiring M, Sioufi J, Marsden K (2016) Type 2M von Willebrand disease – more often misidentified than correctly identified. Haemophilia 22:e145–e155
Favaloro EJ (2017) Utility of the von Willebrand factor collagen binding assay in the diagnosis of von Willebrand disease. Am J Hematol 92:114–118
Favaloro EJ, Pasalic L, Curnow J (2016) Type 2M and Type 2A von Willebrand Disease: similar but different. Semin Thromb Hemost 42:483–497
Franchini M, Lippi G, Veneri D, Targher G, Zaffanello M, Guidi GC (2008) Inherited platelet disorders. Clin Chim Acta 387:1–8
Favaloro EJ (2016) Clinical utility of closure times using the Platelet Function Analyzer (PFA)-100/200. Am J Hematol. doi:10.1002/ajh.24620
Favaloro EJ (2008) Clinical utility of the PFA-100. Semin Thromb Hemost 34:709–733
Favaloro EJ, Lippi G, Franchini M (2010) Contemporary platelet function testing. Clin Chem Lab Med 48:579–598
Lordkipanidzé M (2016) Platelet function tests. Semin Thromb Hemost 42:258–267
Blanchette VS, Key NS, Ljung LR, Manco-Johnson MJ, van den Berg HM, Srivastava A, Subcommittee on Factor VIII, Factor IX and Rare Coagulation Disorders of the Scientific and Standardization Committee of the International Society on Thrombosis and Hemostasis (2014) Definitions in hemophilia: communication from the SSC of the ISTH. J Thromb Haemost 12:1935–1939
Favaloro EJ, Verbruggen B, Miller CH (2014) Laboratory testing for factor inhibitors. Haemophilia 20(Suppl 4):94–98
Bonar RA, Favaloro EJ, Marsden K (2013) External quality assessment of factor VIII inhibitor assays. Semin Thromb Hemost 39:320–326
Adcock DM, Favaloro EJ (2015) Pearls and pitfalls in factor inhibitor assays. Int J Lab Hematol 37(Suppl 1):52–60
Favaloro EJ, Meijer P, Jennings I, Sioufi J, Bonar RA, Kitchen DP, Kershaw G, Lippi G (2013) Problems and solutions in laboratory testing for hemophilia. Semin Thromb Hemost 39:816–833
Peyvandi F, Favaloro EJ (2009) Rare bleeding disorders. Semin Thromb Hemost 35:345–347
Kershaw G, Suresh S, Prellana D, Nguy Y (2012) Laboratory identification of lupus anticoagulants. Semin Thromb Hemost 38:375–384
Plebani M, Sanzari MC, Zardo L (2008) Quality control in coagulation testing. Semin Thromb Hemost 34:642–646
Clinical and Laboratory Standards Institute (CLSI) (2008) Collection, transport and processing of blood specimens for testing plasma-based coagulation assays and molecular haemostasis assays; approved guideline. vol 28, no. 5: CLSI document H21-A5
Lippi G, Salvagno GL, Montagnana M, Lima-Oliveira G, Guidi GC, Favaloro EJ (2012) Quality standards for sample collection in coagulation testing. Semin Thromb Hemost 38:565–575
Adcock Funk DM, Lippi G, Favaloro EJ (2012) Quality standards for sample processing, transportation, and storage in hemostasis testing. Semin Thromb Hemost 38:576–585
Lippi G, Plebani M, Favaloro EJ (2013) Interference in coagulation testing: focus on spurious hemolysis, icterus, and lipemia. Semin Thromb Hemost 39:258–266
Favaloro EJ, McVicker W, Lay M, Ahuja M, Zhang Y, Hamdam S, Hocker N (2016) Harmonizing the International Normalized Ratio (INR): standardization of methodologies and use of novel strategies to reduce inter-laboratory variation and bias. Am J Clin Pathol 145:191–202
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Bonar, R.A., Lippi, G., Favaloro, E.J. (2017). Overview of Hemostasis and Thrombosis and Contribution of Laboratory Testing to Diagnosis and Management of Hemostasis and Thrombosis Disorders. In: Favaloro, E., Lippi, G. (eds) Hemostasis and Thrombosis. Methods in Molecular Biology, vol 1646. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-7196-1_1
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DOI: https://doi.org/10.1007/978-1-4939-7196-1_1
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